Genetic disorders of human neurological and immune function

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The Crow laboratory has two main interests. Firstly, we study a chemical called interferon, which acts as a kind of cellular disinfectant that kills viruses. Like a disinfectant, too much interferon is dangerous. Interferon is normally only produced when cells sense the presence of viral genetic material (DNA and RNA). However, we have identified patients in whom interferon is high in the absence of viral infection, due to the cell misinterpreting our own DNA and RNA as coming from a virus, and the subsequent triggering of interferon production. We are interested in how cells normally avoid such sensing of ‘self’ as ‘non-self’, and what happens when these mechanisms fail. In a second research theme, we study a rare disease of the blood vessels of the brain called Labrune syndrome, which we discovered to arise due to changes in a piece of genetic material called SNORD118. We have developed a zebrafish which does not have SNORD118. Using this model, we want to understand how the blood vessels in our brain are kept healthy, and how problems with the function of SNORD118 cause disease. This could be relevant not only for the development of future treatments for Labrune syndrome, but also in our understanding of more common disorders of the brain blood vessels, such as stroke.We work closely with affected families, using samples that are provided by patients for our research, together with specialised tests and animal models, to better understand and treat the devastating diseases described above.